Said Sarmad, Cooper Chad J, Chowdhury Farhanaz, Nunez Angelica, Quansah Raphael, Davis Harry E
Department of Internal Medicine, Texas Tech University Health Sciences Center, El Paso, TX, U.S.A.
Am J Case Rep. 2013 Oct 21;14:424-9. doi: 10.12659/AJCR.889590. eCollection 2013.
Male, 42 FINAL DIAGNOSIS: Moyamoya disease (MMD) Symptoms: Aphasia • concentration difficulty • dysarthria • personality change
Rare disease.
Moyamoya disease (MMD) was first described in 1957 as "hypoplasia of the bilateral internal carotid arteries." The characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to "something hazy, like a puff of cigarette smoke," which, in Japanese, is Moyamoya. This paper describes the fulminant course of the disease in a Hispanic male involving the corpus callosum.
A 42-year-old Hispanic male with progressive aphasia, slow mentation, and sudden onset of sensorimotor symptoms with gait disturbance was found to have multiple intracranial supratentorial infarcts of variable stages of evolution involving, but not limited to, the anterior corpus callosum, followed by rapid development of further infarcts. Angiography demonstrated right ACA occlusion, left supraclinoid ICA occlusion with a Moyamoya pattern of collateralization, and diffuse arteriopathy. A fulminant course ensued and the patient did not survive the acute phase of ischemic disease.
Moyamoya disease may rarely present in North American Hispanic males, with advanced atypical clinical and imaging features involving the anterior corpus callosum and having a fulminant course.
男性,42岁 最终诊断:烟雾病(MMD) 症状:失语症、注意力不集中、构音障碍、人格改变
罕见病
烟雾病(MMD)于1957年首次被描述为“双侧颈内动脉发育不全”。血管造影中相关异常扩张的侧支血管网络的特征性表现后来被比作“某种模糊的东西,就像一缕香烟烟雾”,在日语中就是烟雾(Moyamoya)。本文描述了一名西班牙裔男性患者中该病累及胼胝体的暴发性病程。
一名42岁的西班牙裔男性,有进行性失语、思维迟缓以及突然出现的感觉运动症状伴步态障碍,被发现有多个颅内幕上梗死灶,处于不同的演变阶段,累及但不限于胼胝体前部,随后进一步梗死灶迅速发展。血管造影显示右侧大脑前动脉闭塞,左侧床突上段颈内动脉闭塞伴烟雾状侧支循环形成,以及弥漫性动脉病变。随后病情呈暴发性发展,患者在缺血性疾病急性期未能存活。
烟雾病在北美西班牙裔男性中可能很少见,具有累及胼胝体前部的晚期非典型临床和影像学特征且病程呈暴发性。